Who Is It For?

XI - The Heart Flashcards Preview

The morphologic and clinical effects of this condition primarily result from progressive damming of blood within the pulmonary circulation. The left ventricle is hypertrophied and dilated, with secondary left atrial dilation. The lungs are heavy and boggy, with perivascular and interstitial transudate, alveolar septal edema, and intra-aleolar edema. Hemosiderin-laden macrophages are present.

This is a hallmark of right sided HF.

Most congenital heart disease arise from faulty embryogenesis during what AOG?

3 - 8 weeks AOG(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 382

12

An abnormal communication between chambers of the heart or blood vessels.

Shunt(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 383

13

These a typically smooth-walled defects near the foramen ovale, usually without associated cardiac abnormalities. Accompanied by right atrial and ventricular dilation, right ventricular hypertrophy and dilation of the pulmonary artery.

Ostium secundum ASD(TOPNOTCH)

14

Reversal of blood flow through a prolonged (left-to-right shunt) due to pulmonary hypertension, yielding right-sided pressures that exceed those on the left side. This causes unoxygenated blood to go into circulation, causing cyanosis.

Eisenmenger syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 383

15

These occur at the lowest part of the atrial septum and can extend to the mitral and tricuspid valves. Abnormalities of the AV Valves are usually present, forming a cleft in the anterior leaflet of the mitral valve or septal leaflet of the tricuspid valve.

Ostium primum ASD(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 384

16

Incomplete closure of the ventricular septum leading to left-to-right shunting. The right ventricle is hypertrophied and often dilated. Diameter of pulmonary artery is increased because of the increased volume by the right ventricle.

Tetralogy of Fallot(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 385

Components of Tetralogy of Fallot.

It is a discordant connection of the ventricles to their vascular outflow. The defect is an abnormal formation of the truncal and aortopulmonary septa. Right ventricular hypertrophy becomes prominent, while the left ventricle becomes somewhat atrophic.

Predominant manifestation of TGA?

Early cyanosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 387

23

Characterized by tubular narrowing of the aortic segment between the left subclavian artery and the ductus arteriosus. DA is usually patent and is the main source of blood to the distal aorta. RV is hypertrophied and dilated, pulmonary trunk is also dilated.

Aorta is sharply constricted by a ridge of tissue at or just distal to the ligamentum arteriosum. Constricted segment is made of smooth muscle and elastic fibers that are continuous with the aortic media, and lined by thickened intima. Ductus arteriosus is closed. Proximally, the aortic arch and its vessels are dilated, LV is hypertrophic.

There is upper extremity hypertension, due to poor perfusion of the kidneys, but weak pulses and low blood pressure in the lower extremities. Claudication and coldness of the lower extremities also present. Enlarged intercostal and internal mammary arteries due to collateral circulation, seen as rib "notching" on xray.

How many percent should the lumen of a blood vessel be obstructed for it to be symptomatic, in the setting of increased demand?

How many percent should the lumen of a blood vessel be obstructed for it to be symptomatic at rest?

90%(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 389

38

Episodic chest pain associated with exertion or some other form of increased myocardial oxygen demand. Pain described as crushing or squeezing substernal sensation which can radiate to left arm. Relieved by rest or vasodilators.

Stable angina(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 390

39

Increasing frequency of pain, precipitated by progressively less exertion, episodes tend to be more intense and longer lasting.

Type of hypertrophybthat develops in patients with volume overload such as aortic valve insufficiency. Characterized by hypertrophy associated with ventricular dilation.

In this disease the left ventricle may exceed 2.0cm in thickness and the heart may weigh >500 grams. Microscopically, myocyte diameter increases, associated with irregular nuclear enlargement and hyperchromasia ("box-car nuclei"), and increased interstitial fibrosis.

It is the failure of a valve to open completely, obstructing forward flow.

Stenosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 401

56

This results from failure of a valve to close completely, thereby allowing reversed flow.

Insufficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 401

57

The hallmark of this disease is heaped-up calcified masses on the outflow side of the cusps, which protrude intonthe sinuses of Valsalva and mechanically impede valve opening. Cusps may become secondarily fibrosed and thickened.

This is characterized by ballooning or hooding of the mitral leaflets. Affected leaflets are enlarged, redundant, thick and rubbery. The tendinous cords are elongated, thinned and occasionally ruptured. Histologically, there is thinning of the fibrosa layer of the valve, accompanied by expansion of the middle spongiosa layer with increased deposition of mucoid material.

Aschoff bodies(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 402

61

These cells have abundant cytoplasm and central nuclei with chromatin arrayed in a slender, wavy ribbon (caterpillar cells) which can be found in all three layers of the heart in rheumatic fever. A component of Aschoff bodies.

Anitschkow cells(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 403

62

Characterized by organization and subsequent scarring, as a sequelae of rheumatic fever. The mitral (or tricuspid) valve is involved, with leaflet thickening, commisural fission and shortening, thickening and fusiong of the chordae tendinae. Fibrous bridging across valvular commisures create "fishmouth" or " buttonhole" deformity.

How many major and/or minor manifestations are needed to diagnose RF?

Endocarditis of previously normal valves, the most common causative agent is S. aureus.

Acute bacterial endocarditis(TOPNOTCH)

68

Serious infection characterized by microbial invasion of heart valves or mural endocardium, often with destruction of the underlying cardiac tissues. The heart valves are friable, bulky and potentially destructive.

Most consistent sign of infective endocarditis.

Fever(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 407

71

Characterized by deposition of sterile, non-inflammatory, nondestructive and small (1mm) masses of fibrin, platelets and other blood components on cardiac valves, along the line of closure of leaflets or cusps.

Sterile vegetations thatvcan develop on the valves of patients with SLE. These are small, granular, pinkish vegetations 1-4mm in diameter and can be located on the undersurface of AV valves, on the cords or endocardium.

The lesions of this disease are distinctive, glistening white intimal plaquelike thickenings on the endocardial surfaces of the cardiac chambers and valve leaflets seen in patients with carcinoid tumors. The lesions are composed of muscle cells and sparse collagen fibers embedded in an acid mucopolysaccharide-rich matrix.

Cardiomyopathy which is characterized by progressive cardiac dilation and contractile dysfunction. The heart is characteristically enlarged and flabby, with dilation of all chambers,the ventricular thickness may be less than, equal to or greater than normal.

Alcohol intake and infection with coxsackie B virus are some of the causes of this cardiomyopathy.

Characterized by myocardial hypertrophy, abnormal diastolic filling and ventricular outflow obstruction. The heart is thick-walled, heavy and hypercontracting. There is an assymetrical septal hypertrophy described as "banana-like". Histologically, there is severe myocyte hypertrophy and disarray with interstitial fibrosis.

A common cause of sudden death in young athlethes.

The ventricles are of approximately normal size or slightly enlarged, the cavities not dilated, and the myocardium is firm. Biatrial dilation is common. Microscopically, there is interstitial fibrosis, varying from minimal to patchy to extensive and diffuse.

Myocarditis distinctive by virtue of parasitization of scattered myofibers by trypanosomes accompanied by an inflammatory infiltrate of neutrophils, lymphocytes, macrophages and occasional eosinophils.

Chagas myocarditis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 414

86

Viruses which account for most cases of myocarditis.

Coxsackie A and B(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 414

87

Type of pericarditis found in patients with uremia or viral infection. The exudate imparts an irregular apperance to the pericardial surface (bread and butter pericarditis).

The most common tumor of the heart.

Metastatic tumor(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 417

Most common primary tumor of the adult heart.

Myxoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 417

Major clinical manifestations of this cardiac tumor are due to valvular "ball-valve" obstruction, embolization or a syndrome of constitutional symptoms.

Myxoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 418

97

The most frequent primary tumor of the heart in infants and children. These are generally small gray-white myocardial masses composed of a mixed population of cells, the most characteristic of which are large, rounded or polygonal cells containing numerous glycogen-laden vacuoles separated by strands of cytoplasm, so-called spider cells.

Group of congenital heart disease characterized by increase pulmonary blood flow but are not initially associated with cyanosis

Most common genetic cause of congenital heart disease

Trisomy 21 (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 533

104

A 24 y/o female complained of shortness of breath and orthopnea lasting several days. The patient reported having diagnosed since childhood as having "hole in the heart." Physical exam revealed holosystolic murmur most audible in the left parasternal area accompanied by thrill. Rales were heared in the bilateral lower lung field. The most likely cause of her condition:

First 6-12 hours(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 545

Contraction bands(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 546

116

A 62 y/o obese male had sudden onset of heaviness in the chest, associated with diaphoresis and dyspnea which started 3 hours prior to consult at the ER. The biomarkers that are most sensitive and specific of myocardial damage that you will request:

Troponins I and T (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 547

117

A 72 y/o female experienced chest pain and hypotension. A posterior transmural infarct was suspected. Most common complications in this type of infarct

Free wall rupture, expansion, mural thrombi, and aneurysm are common in what type/location of infarct?

Most common cause of rhythm disorder

Ischemic injury(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 550

120

A 24 y/o female presents with history of recurrent fever and joint pains accompanied by ECG changes and increased ASO titer in the past 2 years. Physical examination reveals cardiac murmur. What is the clinical impression?

A 5 wk old infant presents with tachypnea, diaphoresis, and difficulty feeding. A harsh, continuous, machinery-like murmur was noted upon auscultation. What is the most likely diagnosis?

Patent ductus arteriosus(TOPNOTCH)

127

Presents with hypertension in the upper extremities, and manifestations of arterial insufficiency such as claudication and coldness. Produce a radiographical visible erosion (notching) of the undersurfaces of the ribs.

Predominant manifestations of RF

Classic hallmark of Infective endocarditis

A 72 year old hypertensive female last seen apparently well 3 days ago, is found dead in her bathroom with rigor mortis and no signs of foul play. At autopsy, her heart showed left ventricular hypertrophy and a pale tan area at the anteroseptal wall. There are no thrombi in the heart chambers. The valves are unremarkable. Microscopic examination of the pale area showed well-established granulation tissue with new blood vessels and collagen deposition. Neutrophils are rare. She died of (A) an MI that occured 1 hour prior to demise (B) an MI 12 hours prior to demise (C) an MI 2 days prior to demise (D) something else entirely

In hypertensive heart disease, there is concentric thickening of the left ventricular wall. A concomitant left atrial dilatation may also be seen due to (A) volume overload from a ventricle with narrowed lumen (B) pressure overload from a ventricle with narrowed lumen (C) cytokines secreted by hypertrophic ventricular myocytes cause atrophy of atrial myocytes (D) cytokines secreted by hypertrophic ventricular myocytes cause metaplasia of atrial myocytes

What feature in a stenotic aortic valve suggests rheumatic valvular disease, rather than calcific aortic stenosis? (A) bicuspid valve (B) masses of calcium on the outflow side of cusps (C) fibrotic cusps (D) fusion of the commmissures

A 20 year old asymptomatic female is found to have a midsystolic click on her preemployment physical examination. A 2D echo showed mitral valve prolapse. The involved leaflet would show (A) numerous fibroblasts with and dense collagen deposition (B) thinning of the fibrosa layer and myxoid expansion of the spongiosa layer (C) deposition of amorphous material that shows apple-green birefringence when stained with Congo red (D) fibrous stroma with gland-like structures secreting mucin

A 5 year old male who had a sore throat 3 weeks ago develops fever and joint pains. Auscultation revealed a friction rub, and ASO titers are increased. Which of the following is expected in the patient? (A) friable vegetations on the mitral valve containing fibrin, neutrophils and gram-positive cocci (B) small vegetations on the mitral valve with abundant eosinophils (C) myocardium with circumscribed aggregates of mononuclear cells and macrophages with prominent nucleoli (D) myocardium with poorly-circumsccribed aggregates of multinucleated giant cells

A 19 year old football player dies suddenly during one training session. At autopsy, his heart showed myocardial hypertrophy with disproportionate thickening of the septum, and a narrowed left ventricular lumen. Microscopic examination showed myocyte hypertrophy, myofiber disarray, and interestitial fibrosis. These findings are due to (A) a mutation in one of his genes encoding sarcomeric proteins (B) a silent Coxsackie virus B infection (C) an undisclosed 3 year history of alcohol intake (D) anabolic steroids he has been taking for 6 months

A 34 year old female on routine checkup is found to have a diastolic murmur. 2D echo showed a pedunculated 3 cm mass in her left atrium attached to the atrial septum. She has no other known masses on workup. She undergoes heart surgery where the atrial mass is resected. Which of the following is its most likely histology? (A) stellate cells admixed with endothelial and fibroblastic cells embedded in an abundant extracellular matrix (B) sheets of large polygonal cells containing glycogen-containing vacuoles arranged around a central nucleus (C) fascicles of fibroblasts and interspersed collagen bundles (D) sheets of pleomorphic cells lining vascular spaces, some with intracytoplasmic lumens, with atypical mitoses and areas of necrosis